Cleft Lip and Palate

1.Cleft lip(cheiloschisis) is a congenital anomaly that occurs at a rate of 1 in 800 births.

a.If the cleft does not affect the palate structure of the mouth it is referred to as cleft lip.

b.Cleft lip is formed in the top of the lip as either a small gap or an indentation in the lip (partial or incomplete cleft) or it continues into the nose (complete cleft)

c.Cleft lip can be unilateral or bilateral.

d.It is due to the failure of fusion of the maxillary and medial nasal processes (formation of the primary palate).

2.Cleft palate (palatoschisis) is a congenital anomaly that occurs in approximately 1 of every 2000 births, and it is more common in boys than girls.

a.It is a condition in which the two plates of the skull that form the hard palate (roof of the mouth) are not completely joined.

b.It ranges in severity from soft palate involvement alone to a defect including the hard palate and portions of the maxilla.

c.Cleft palate may or may not be associated with cleft lip.

3.Children with these structural disorders may have associated dental malformations, speech problems, and frequent otitis media, the latter resulting from improper functioning of the Eustachian tubes.

B. Etiology

1.Many factors are associated with the development of cleft lip and cleft palate, and cleft lip with or without cleft palate is developmentally and genetically different from isolated cleft palate.

2.Most cases appear to be consistent with the concept of multifactorial inheritance as evidenced by an increase incidence in relatives and monozygotic twins.

C. Pathophysiology

1.During embryonic development the lateral and medial tissues forming the upper lip palates fuse between weeks 7 and 8 of gestation; the palatal tissues forming the hard and soft palates fuse between weeks 7 and 12 gestation.

2.Cleft lip and cleft palate result when these tissues fail to fuse.

D. Assessment findings

1.Clinical manifestations

a.Cleft lip and cleft palate are readily apparent at birth. Careful physical assessment should be performed to rule out other midline birth defects.

b.Cleft lip and cleft palate appear as incomplete or complete defects, and may be unilateral or bilateral.

2.Laboratory and diagnostic study findings. Obstetric ultrasound will reveal cleft lip while the infant is in utero.

E. Nursing management

1.Assess for problems with feeding, breathing parental bonding, and speech.

2.Ensure adequate nutrition and prevent aspiration.

a.Provide special nipples or feeding devices (eg, soft pliable bottle with soft nipple with enlarged opening) for a child unable to suck adequately on standard nipples.

b.Hold the child in a semiupright position; direct the formula away from the cleft and toward the side and back of the mouth to prevent aspiration.

c.Feed the infant slowly and burp frequently to prevent excessive swallowing of air and regurgitation.

d.Stimulate sucking by gently rubbing the nipple against the lower lip.

3.Support the infant’s and parents’ emotional and social adjustment.

a.Help facilitate the family’s acceptance of the infant by encouraging the parents to express their feelings and concerns and by conveying an attitude of acceptance toward the infant.

a.Depending in the defect and the child’s general condition, surgical correction of the cleft lip usually occurs at 1 to 3 months of age; repair of the cleft palate is usually performed between 6 and 18 months of age. Repair of the cleft palate may require several stages of surgery as the child grows.

b.Early correction of cleft lip enables more normal sucking patterns and facilitates bonding. Early correction of cleft palate enables development of more normal speech patterns.

c.Delayed closure or large defects may require the use of orthodontic appliances.

c.Position the child with cleft lip on her back, in an infant seat, or propped on a side to avoid injury to the operative site; position the child with a cleft palate on the abdomen to facilities drainage.

d.Clean the suture line and apply an antibacterial ointment as prescribed to prevent infection and scarring. Monitor the site for signs of infection.

e.Use elbow restraints to maintain suture line integrity. Remove them every 2 hours for skin care and range-of-motion exercises.

f.Feed the infant with a rubber-tipped medicine dropper, bulb syringe, Breck feeder, or soft bottle-nipples, as prescribed, to help preserve suture integrity. For older children, diet progresses from clear fluids; they should not use straws or sharp objects.

g.Attempt to keep the child from putting tongue up to palate sutures.

h.Manage pain by administering analgesic as prescribed.

6.Provide child and family teaching.

Demonstrate surgical wound care.

Show proper feeding techniques and positions.

Explain that temperature of feeding formulas should be monitored closely because new palate has no nerve endings; therefore; the child can suffer a burn to the palate easily and without knowing it.

Explain handling of prosthesis if indicated.

Stress the importance of long-term follow up, including speech therapy, and preventing or correcting dental abnormalities.

Discuss the need for, at least, annual hearing evaluations because of the increased susceptibility to recurrent otitis. The child may require myringotomy and surgical placement of drainage tubes.